Abstract 15986: Comparative Outcomes in Marfan and Non-Marfan Aortopathy
Heritable aortopathy causes thoracic aneurysm and dissection (TAAD) in younger adults and survival predictors are likely to differ from older hypertensive disease. From 1988 to 2011, 211 Marfan (revised Ghent criteria) and 288 non-Marfan TAAD patients with aortic dilation (Z score > 2) were followed in a dedicated clinic for 10 ± 8 years (follow-up 98% complete). Hypertensive and atherosclerotic disease and bicuspid valve are excluded. 136 (64%) Marfan and 161(56%) non-Marfan TAAD patients had family history of aortic disease; 72 (34%) Marfan and 117 (41%) non-Marfan TAAD patients had family history of aortic dissection.
Survival free of cardiac death and aortic dissection was compared between groups (Kaplan-Meier). Outcome predictors were identified by Cox proportional hazards model (SPSS v.19).
Cardiac death or dissection occurred in 49 (23%) Marfan and 68 (24%) non-Marfan TAAD; risk increased with aortic diameter (p<0.001); 40-44 mm = 14.7%, 45-49 mm = 31.1%, 50-54 mm = 32.6%, ≥ 55 mm = 57.1%. Mean survival free of death/dissection was 60.8±2.8 years (Marfan) and 66.5±1.5 years (non-Marfan) (p=0.002) and independent predictors were family history of aortic dissection and aorta size ≥45 mm.
Both Marfan and non-Marfan aortopathy have high risk of death/dissection and major risk predictors are family history and aortic diameter. Both Marfan and non-Marfan TAAD patients with family history of aortic dissection should be considered for aortic repair when aorta size ≥45 mm.
- © 2012 by American Heart Association, Inc.